224 - RA Flashcards
Where is the primary site of RA?
Synovium
What changes occur to the synovium in RA?
- Pannus formation - thickened, inflamed
- erosion of bone, cartilage and ligament
- Excess synovial fluid
How common is RA?
0.5-1% of population
What risk factors are there for RA?
Smoking
Genetic - assoc. with HLA-DR4 and PTPN 22
What is the usual presentation for RA?
Gradual onset, in small peripheral joints
Morning stiffness, swelling in joints + hands, symmetrical
What is the pattern of RA joints?
Usually PIPs, MCPs and wrist
What changes can be seen in the hand of someone with RA?
Ulner deviation of the fingers
Dorsal interosseous wasting
Swan neck deformities
Boutonnier deformity
What can be seen on Xray in RA?
Periarticular osteopenia (looks darker)
Periarticular errosions
Reduced joint space
Ulner deviation + subluxation
In RA what are the systemic effects it has?
Nodules - s/c gristle
Vasculitis
Cardiac (IHD, pericarditis, valvular disease)
Respiratory (Effusions, fibrosis, nodules)
Haem (Anaemia of chronic disease, thrombocytosis)
Neuro (entrapement neuropathies, atlanto-axial subluxation)
Occular (Episcleritis, scleromalacia)
Amyloidisis - builds up in tissues eg. tongue
How do you diagnose RA?
Clincial signs
Bloods - ESR, CRP, Rheumatoid Factor, Anti-CCP
Imaging - US, Xray
Which is better to diagnose RA - RF or Anti CCP?
Anti-CCP - more specific to RA (but not as sensitive, only 70% of RA have it)
RF is good as 80% with RA are +ve, but 10% of normal population also +ve
What is the treatment of RA?
Analgesia
NSAIDS
Steroids
DMARDS (Methotrexate, Sulphasalazines, Leflunomide)
Biologics (anti-TNF a - infliximab, Anti CD20 - Rituximab)
What characterises connective tissue diseases?
Inflammation, Fibrosis, Thrombosis
What are the common symptoms of connective tissue diseases?
Fatigue, malaise, fever Depression Arthralgia/myalgia Weight loss Lymphadanopathy
What clinical features might be found in someone with connective tissue disease?
Raynauds Inflammatory arthritis Skin rashes Muscle weakness Pleurisy Vascular events Recurent foetal loss
What associations are there with connective tissue disease?
HLA
Ethnic susceptibilities
Name 4 connective tissue diseases
SLE : systemic lupus erythematosis
Inflam muscle disease - polymyolitis or dermatomyolitis
Scleroderma
Sjorgrens syndrome
What specific signs are there in SLE?
Butterfly rash - photosensitive Arthritis Oral ulcers Alopecia Serositis
What are the autoantibodies associated with SLE?
ANA (100% +ve),
ds DNA (50% +ve),
anti-sm (15%),
anti-Ro/La (20%)
What treatment options are there for SLE?
NSAIDS
Hydroxychloroquine to myalgia
Prednisalone for arthritis
What is scleroderma otherwise known as?
Systemic sclerosis
What are the features of scleroderma?
Fibrotic skin tightening (proximal to elbow)
Can be limited, diffuse or localised
In diffuse scleroderma what autoantibody is seen?
anti-scl 70
What are you at increased risk of with diffuse scleroderma?
Pulmonary fibrosis
Renal Crisis
What is another name for limited scleroderma? What does it mean?
CREST syndrome C-alcinosis R-aynauds E-osophageal dysmotility S-clerodactyly T-alangectasia
What autoantibodies are present in limited scleroderma/CREST?
Anti-centemere autoantibodies
What are the symptoms of Sjorgrens Syndrome?
Dry eyes + mouth
Parotid swelling
What autoantibodies are seen in Sjorgrens syndrome?
Ro (ssa) / La (ssb) autoantibodies
ANA
RF
Which connective tissue disease puts you at higher risk for lymphoma?
Sjorgrens
What is polymyositis?
Direct inflammation of the muscle itself
Symmetrical prox muscle weakness
What systemic problems can you see with an inflammatory muscle disease?
Heliotrope rash - around eyes and eyelids
Guttrons papules - dry scaly skin on knuckles
Mechanics hands - dry scaly skin on tips of fingers
What enzyme is elevated in inflammatory muscle disease?
CPK - elevated muscle enzyme
How do you treat polymyositis?
Steroids
Immunosuppressants
What is vasculitis?
Inflammation within or through a vessel wall - causing an impairment in blood flow + integrity of the vessel - haemorrhage or infarction can occur.
What is PMR and what is it associated with?
Polymyalgia Rheumatica
Assoc. with GCA - Giant cell Arthritis
What are the features of PMR? What age does it affect?
Affects middle ages/ elderly
Pain + stiffness in neck, shoulder, pelvic girdle.
Worse in morning, improves with activity
What systemic effects are there in PMR?
Malaise, weight loss, flue-like
What blood tests are raised in Vasculitis?
ESR
CRP
What is Giant cell arthritis?
affects large vessels (with internal elastic components)
eg. aortic arch and branches
Causes: headaches, jaw claudication, fatigue, visual disterbances
If Giant cell arthritis is left untreated what are they at risk of?
Blindness
Stroke
MI
Necrosis
What investigation can be done to confirm giant cell arthritis?
Temporal artery biopsy
What can be given to treat GCA?
High dose prednisolone
Aspirin
Osteoporosis prophylaxis
Name 4 types of small vessel vasculitis?
Wegners
Polyangiitis
Polyarthritis Nodosa
Churg-Straus
What are the general symptoms + systemic effects seen in Small vessel vasculitis?
Malaise, fever, weight loss
Skin - purpura, ulcers, infarction ENT - Epistaxis, sinusitis, deafness Resp- Cough, haemoptysis, dyspnoea GI- ulcers, pain, diarrhoea Neuro - Sensory/motor impairments Cardiac - chest pain, MI Renal - Renal failure
What investigations are important in small vessel vasculatis?
Inflammatory markets (FBC, ESR, CRP) ANCA +ve - antineurophil cytoplasmic antibody
(can look for specific antigen involvement, eg. PR3 in wegners)
What are the core treatment approaches for small vessel vasculitis?
Induce remission - steroids, cyclophosphamide, biologics (anti-TNFs or rituximab)
Maintain remission - Azathioprine, methotrexate, biologics, mycophenolate mofetil (immunosuppressant)
What is Wegner’s / Granulomatous polyangiitis
Destructive vasculitis, damages midline structures: nose, trachea, lungs, eyes, glomerulonephritis
Assoc with ANCA PR3
What is Polyarthritis nodosa?
Rare vasculitis, aneurysms in medium sized vessels Rash, mononeuritis multiplex High BP Testicular pain GI infarction
What is microscopit polyangiitis?
Rare vasculitis
ANCA MP0
What is churg strauss?
Weakly ANCA associated vasculitis
Eosinophilia
Linked to adult onset asthma
What is antiphospholipid syndrome?
Recurrent vascular thrombosis, pregnancy loss and thrombocytopoemia.
Assoc. with antiphosopholipid antibodies
Treat with warfarin.